title: [Pemphigus: a review].
abstract: BACKGROUND
  Pemphigus is a rare autoimmune bullous disorder. Numerous treatment regimens have been proposed in the literature.
  
  
  OBJECTIVE
  To assess the efficacy and tolerance of treatment regimens proposed in pemphigus vulgaris (PV) and pemphigus foliaceus (PF), from a systematic review of the literature.
  
  
  METHODS
  Randomized control trials have been identified using the PubMed and Embase databases up to April 2009. Uncontrolled prospective and retrospective studies have also been analyzed.
  
  
  RESULTS
  Eleven randomized control trials having included a total number of 421 patients (377 PV, 44 PF) have been analyzed. Most studies had a limited statistical power due to the rather low number of cases included. Results from ten different treatment regimens have been analyzed: different dosages of prednisone and prednisolone, pulse intravenous dexamethasone, azathioprine, cyclophosphamide, cyclosporine, dapsone, mycophenolate mofetil, plasmapheresis, topical applications of epidermal growth factor (EGF), and intravenous immune globulins (IVIG). Inclusion criteria were: (i) consecutive patients in nine studies, (ii) patients who did not respond to low doses of corticosteroids in one study, and (iii) patients with relapsing type of pemphigus in one study. None of these studies allowed identifying the best effective and well tolerated regimen. Mycophenolate mofetil was more effective than azathioprine for disease control (from one study; n=40; OR=0.72; 95% CI=0.52-0.99). However, no difference in the rate of clinical remission was evidenced between these drugs. Azathioprine and cyclophosphamide seem to have a corticosteroid sparing effect.
  
  
  CONCLUSIONS
  Data from the literature did not allow identifying the best therapeutic regimen, mainly because of the lack of statistical power of most studies. The usefulness of immunosuppressant added to systemic corticosteroids as the first line of treatment is not clearly established.
authors.affiliation: Clinique dermatologique, hôpital Charles-Nicolle, université de Rouen, 1 rue de Germont, Rouen cedex, France. Pascal.Joly@chu-rouen.fr
authors.forename: P
authors.initials: P
authors.lastname: Joly
authors.forename: C
authors.initials: C
authors.lastname: Sin
mesh_terms: D000305:Adrenal Cortex Hormones / Q000008:administration & dosage / Q000627:therapeutic use; D003131:Combined Modality Therapy; D004359:Drug Therapy, Combination; D004815:Epidermal Growth Factor / Q000008:administration & dosage / Q000627:therapeutic use; D017612:Gold Compounds / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D015201:Meta-Analysis as Topic; D015337:Multicenter Studies as Topic / Q000706:statistics & numerical data; D009536:Niacinamide / Q000008:administration & dosage / Q000627:therapeutic use; D010257:Paraneoplastic Syndromes / Q000188:drug therapy; D010392:Pemphigus / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D010951:Plasma Exchange; D011446:Prospective Studies; D016032:Randomized Controlled Trials as Topic / Q000706:statistics & numerical data; D012008:Recurrence; D012189:Retrospective Studies; D013752:Tetracycline / Q000008:administration & dosage / Q000627:therapeutic use
chemical_list: D000305:Adrenal Cortex Hormones; D017612:Gold Compounds; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D009536:Niacinamide; D004815:Epidermal Growth Factor; D013752:Tetracycline